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A rare cause of congestive heart failure1
The Journal of Emergency Medicine, Vol. 20, No. 2, pp. 153–157, 20001 Copyright © 2001 Elsevier Science Inc. Printed in the USA. All rights reserved 0736-4679/01 $–see front matter
PII S0736-4679(00)00305-X
Case Presentations of the Harvard Emergency Medicine Residency
A RARE CAUSE OF CONGESTIVE HEART FAILURE Alan M. Kumar,
MD,*
Laura J. Bontempo,
MD,*†
Eric S. Nadel,
MD,*†‡
and David F.M. Brown,
MD*‡
*Division of Emergency Medicine, Harvard Medical School; †Department of Emergency Medicine, Brigham and Women’s Hospital; and ‡Department of Emergency Medicine, Massachusetts General Hospital, Boston, Massachusetts Reprint Address: Eric S. Nadel, MD, Department of Emergency Medicine, Brigham and Women’s Hospital, 75 Francis Street, Boston, MA 02115
Dr. Alan Kumar: Today’s case is that of a 45-year-old woman who presented to our Emergency Department (ED) complaining of increased abdominal swelling and a 10-pound weight loss over a period of 4 weeks. The patient noted progressive shortness of breath that was worse when lying flat, and lower extremity edema. The patient had no other complaints. She denied fevers, chills, sweats, chest pain, nausea, vomiting, or abdominal pain. She had no positive medical history, was on no medications, and had no medication allergies. Are there any questions regarding the initial history? Dr. Andrew McAfee: The most concerning information thus far is the 10-pound weight loss, which may be caused by a chronic process such as malignancy or tuberculosis. In a woman of this age, with minimal focal symptoms other than abdominal distension, one has to be concerned about ovarian pathology. All of the other symptoms appear to be subtle. As you were talking with the patient, did she appear in any distress? Dr. Kumar: The patient appeared slightly tachypneic, and in no distress. Dr. Christopher Richards: Shortness of breath with an element of orthopnea and lower extremity edema is of concern for congestive heart failure (CHF). Did she have any risk factors for CHF or coronary artery disease (CAD)? Dr. Kumar: She did not smoke cigarettes, denied excessive alcohol use, and had no family history of hypertension, CAD, or diabetes.
Dr. Laura Macnow: Had the patient noted any change in her exercise tolerance, which may be indicative of coronary ischemia? Dr. Kumar: The patient did note generalized increased fatigue; however, she was not able to say specifically whether her physical endurance was affected. On physical examination, the patient was a thin woman, with a protuberant abdomen and an increased respiratory rate. She was non-toxic in appearance. The vital signs were: temperature of 36.5°C (97.6°F); heart rate 105 beats per minute; blood pressure 123/85 mm Hg; respiratory rate 28 breaths per minute; and an oxygen saturation of 96% on room air. Pupils were equally reactive, and sclerae were icteric. The mucous membranes were moist and appeared jaundiced. Jugular venous distention was measured at 12 cm. The thyroid gland was not palpable. There were crackles halfway up the lung fields bilaterally, and the cardiac examination revealed a tachycardic rate, regular rhythm, and a three of six holosystolic murmur radiating to the axilla. The murmur was loudest at the apex and was noted to be intermittent. The abdomen was distended, soft, and nontender. There was a fluid wave, and the liver was percussed to 13 cm. The rectal examination revealed good tone, no tenderness, and the hemoccult test was negative. There was 2⫹ pitting edema to the knees, and good distal pulses. There were no rashes. The patient was alert and oriented on neurologic examination, with normal reflexes, gait, and cranial nerves.
Case Presentations of the Harvard Emergency Medicine Residency is coordinated by David F. M. Brown, Eric S. Nadel, MD, of the Harvard Medical School, Boston, Massachusetts
RECEIVED: 23 October 2000; ACCEPTED: 3 November 2000 153
MD,
and
154
A. M. Kumar et al.
Figure 1. EKG reveals sinus tachycardia with no evidence of ischemia.
Dr. Laura Bontempo: I agree that the weight loss is a concern for carcinoma. In light of the findings of painless jaundice, I am particularly concerned about a gastrointestinal malignancy blocking the biliary tree outlet. Had she noticed the jaundice or a change in urine color? Dr. David Brown: Congestive heart failure in a young patient with a new heart murmur has to raise the possibility of endocarditis. Had she had any recent dental work? Although the patient has no fever or peripheral signs of endocarditis, blood cultures should be drawn. Dr. Kumar: The patient noticed her eyes becoming yellow over the past 2 weeks. She had not noticed a change in urine color, although she did note a decreased urine output. She has not had any recent dental work. Dr. J. T. Nagurney: The presentation is most consistent with right and left-sided heart failure. Valvular heart disease, supported by the mitral regurgitation murmur, is most likely, but one also should consider dilated cardiomyopathy secondary to alcohol, cocaine, viral, hyperthyroid, or ischemic etiologies. Less likely causes of CHF include constrictive pericarditis and infiltrative disease such as amyloidosis and sarcoidosis. Chagas disease
might be considered if the patient had traveled to South America. Dr. Nadel: Are there any thoughts as to priorities in the management of this patient in the ED? Dr. Ron Walls: The most important thing is to establish that there is no immediately life-threatening process and, based on the presentation, this does not appear to be the case. In the ED, the patient needs a complete blood count (CBC) drawn to evaluate for anemia and eosinophilia, a metabolic panel looking for renal dysfunction or electrolyte abnormalities, an electrocardiogram (EKG), and a chest radiograph (CXR). I would draw three sets of blood cultures, separated in time and location, to evaluate for endocarditis. As far as treatment, I would administer furosemide intravenously. Dr. Bontempo: Given the painless jaundice, I would also consider an imaging test to look at the hepatobiliary system. You could proceed with an ultrasound or a computed tomography (CT) scan. The CT scan would give more information regarding other abdominal pathology, but the ultrasound would be better for assessing biliary tree dilatation. What did you do?
Congestive Heart Failure
155
Figure 3. Abdominal CT scan reveals ascites.
Figure 2. Chest X-ray reveals pulmonary edema and cardiomegaly.
Dr. Kumar: The patient was placed on a cardiac monitor, and the laboratory tests mentioned above were obtained, as well as tropinin I for the possibility of silent infarction, and a thryroid-stimulating hormone level was sent. The EKG (Figure 1) showed sinus tachycardia and right axis deviation. The CXR (Figure 2) revealed pulmonary edema and cardiomegaly. Laboratory values showed normal electrolytes and renal function and normal complete blood count with a hematocrit of 36.6% and white blood cell count of 6.0 K/mm3 with normal differential. The liver function panel revealed an alkaline phosphatase of 94 U/L, ALT 18 U/L, AST 34 U/L, and total bilirubin 3.6 mg/dL. The troponin-I was 0.05 ng/ mL. An abdominal/pelvic CT scan was performed (Figure 3), showing massive ascites, but no other intrabdominal pathology. Dr. Brown: Without evidence of pathology in the abdomen on CT scan, the ascites is likely secondary to hepatic congestion due to heart failure. Cardiac pathology, including endocarditis and an infiltrative process, must remain high on the differential diagnosis. As an inpatient, an echocardiogram should be obtained to search for valvular vegetations and to assess the ejection fraction. Rheumatic fever is a known risk factor for endocarditis, as is intravenous drug use. Is there any such history, and did you start antibiotics? Dr. Kumar: The patient denied any history of intravenous drug use. There was no known history of rheumatic fever. After blood cultures were drawn, vancomycin and gentamicin were given in the ED. The antibiotics
were targeted to cover Streptococcus viridans and Staphylococcus aureus. After the initiation of antibiotic therapy and diuretics, the patient was admitted to the medical service for further evaluation. A transthoracic echocardiogram revealed an ejection fraction of 57%, mild left ventricular enlargement, moderate aortic insufficiency, severe mitral regurgitation, and an echodensity on the posterior leaflet of the mitral valve. (Figure 4) The findings were suggestive of a chronic process, but endocarditis could not be ruled out. Antibiotics were continued until all six bottles of blood cultures remained negative for 48 h. Laboratory studies were directed at viral and rheumatologic causes of the valvular damage, and presumed chronic vegetation. Additional laboratory tests included an erythrocyte sedimentation rate (ESR), antistreptolysin-O (ASLO), and antinuclear anitibody (ANA). HACEK blood cultures were sent as well. These blood cultures test for Haemophilus species other than influezae as well as Actinobacillus, Cardiobacterium, Eikenella, and Kingella species. They account for up to 5% of patients with endocarditis and require special media in a carbon dioxide atmosphere for incubation. They are commonly referred to as the HACEK organisms (1). The ESR returned at 11, the ANA and ASLO were both negative, and the HACEK blood cultures were also negative. The patient continued to receive diuretics throughout her evaluation. The patient was brought to the operating room on hospital day 7 for a mitral valve replacement and aortic valve repair. Intra-operatively, the aortic valve was felt to have too much damage for successful repair, and both the mitral and aortic valves were replaced with prostheses. The removed valves were sent to the microbiology laboratory for cultures, which
156
A. M. Kumar et al.
Figure 4. Echocardiogram reveals an echodensity (mass) on the posterior leaflet of the mitral valve.
were negative. Pathology evaluation showed evidence of high-grade spindle cell sarcoma with muscle differentiation suggestive of a primary cardiac sarcoma. A Resident: Is jaundice common in patients with right heart failure? Dr. Brown: In moderate to severe heart failure, scleral icterus may be due to hepatic congestion. In this case, there also may be some contribution to the total bilirubin due to hemolysis on the abnormal heart valves. Dr. Nadel: This is obviously a rare diagnosis, not often seen in the ED. Please tell us more about it. Dr. Kumar: Cardiac tumors present with a wide array of symptoms mimicking the more common forms of heart disease, including chest pain, dysrhythmias, heart failure, syncope, pericarditis, or cardiac tamponade (2). Cardiac myxomas, although rare, are the most common type of cardiac tumor. They are histologically benign but still are life-threatening because of the possibility of obstruction, valvular damage, or embolus. Cardiac sarcomas are even rarer. They generally cause a rapidly progressive clinical deterioration over weeks to months with hemodynamic compromise, local invasion, or distant metastases (3). Diagnosis of cardiac tumors is best done by echocardiography, although differentiation of the type of tumor must be done by pathology. Not surprisingly, these tumors are often mistaken for endocarditis or collagen vascular disease. Cardiac magnetic resonance imaging can also better characterize the size, shape, and surface characteristics of the tumor (4). Ther-
apy is more successful for cardiac myxomas if they are detected early. Treatment involves surgical excision. Sarcomas generally have spread too far by the time of clinical presentation for surgical excision (5). There are a few case reports of palliation with the combination of surgery, radiotherapy, and chemotherapy, but results are generally poor. A few centers are experimenting with cardiac transplantation as a possibly curative measure for sarcomas (6). Dr. Bontempo: What was the patient’s post-operative course, and what is her long-term treatment plan? Dr. Kumar: The patient developed third-degree anterior-ventricular block on post-operative day 2 and had a pacemaker placed 2 days later by the electrophysiology service. A repeat echocardiogram showed an ejection fraction of 47%. The patient was evaluated by the oncology service and was offered palliative chemotherapy with adriamycin, and a referral to a cardiac transplant center for possible cardiac transplant. The rest of her hospitalization was uneventful, and the patient was discharged home on post-operative day 12.
REFERENCES 1. Meyer DJ, Gerding DN. Favorable prognosis of patients with prosthetic valve endocarditis caused by gram-negative bacilli of the HACEK group. Am J Med 1998;85:104. 2. Pucci A, Gagliardotto P, Zanini C, Pansini S, di Summa M, Mollo F. Histopathologic and clinical characterization of cardiac myx-
Congestive Heart Failure oma: review of 53 cases from a single institution. Am Heart J 2000;140:134 – 8. 3. Pins MR, Ferrell MA, Madsen JC, Piubello Q, Dickersin GR, Fletcher CD. Epithelioid and spindle-celled leiomyosarcoma of the heart. Report of 2 cases and review of the literature. Arch Pathol Laboratory Med 1999;123:782– 8. 4. Grebenc ML, Rosado de Christenson ML, Burke AP, Green CE,
157 Galvin JR. Primary cardiac and pericardial neoplasms: radiologicpathologic correlation. Radiographics 2000;20:1073–103; quiz 1110 –1, 1112. 5. Schaff HV, Mullany CJ. Surgery for cardiac myxomas. Seminars Thoracic Cardiovascular Surg 2000;12:77– 88. 6. Michler RE, Goldstein DJ. Treatment of cardiac tumors by orthotopic cardiac transplantation. Semin Oncol 1997;24:534 –9.
PII S0736-4679(00)00305-X
Case Presentations of the Harvard Emergency Medicine Residency
A RARE CAUSE OF CONGESTIVE HEART FAILURE Alan M. Kumar,
MD,*
Laura J. Bontempo,
MD,*†
Eric S. Nadel,
MD,*†‡
and David F.M. Brown,
MD*‡
*Division of Emergency Medicine, Harvard Medical School; †Department of Emergency Medicine, Brigham and Women’s Hospital; and ‡Department of Emergency Medicine, Massachusetts General Hospital, Boston, Massachusetts Reprint Address: Eric S. Nadel, MD, Department of Emergency Medicine, Brigham and Women’s Hospital, 75 Francis Street, Boston, MA 02115
Dr. Alan Kumar: Today’s case is that of a 45-year-old woman who presented to our Emergency Department (ED) complaining of increased abdominal swelling and a 10-pound weight loss over a period of 4 weeks. The patient noted progressive shortness of breath that was worse when lying flat, and lower extremity edema. The patient had no other complaints. She denied fevers, chills, sweats, chest pain, nausea, vomiting, or abdominal pain. She had no positive medical history, was on no medications, and had no medication allergies. Are there any questions regarding the initial history? Dr. Andrew McAfee: The most concerning information thus far is the 10-pound weight loss, which may be caused by a chronic process such as malignancy or tuberculosis. In a woman of this age, with minimal focal symptoms other than abdominal distension, one has to be concerned about ovarian pathology. All of the other symptoms appear to be subtle. As you were talking with the patient, did she appear in any distress? Dr. Kumar: The patient appeared slightly tachypneic, and in no distress. Dr. Christopher Richards: Shortness of breath with an element of orthopnea and lower extremity edema is of concern for congestive heart failure (CHF). Did she have any risk factors for CHF or coronary artery disease (CAD)? Dr. Kumar: She did not smoke cigarettes, denied excessive alcohol use, and had no family history of hypertension, CAD, or diabetes.
Dr. Laura Macnow: Had the patient noted any change in her exercise tolerance, which may be indicative of coronary ischemia? Dr. Kumar: The patient did note generalized increased fatigue; however, she was not able to say specifically whether her physical endurance was affected. On physical examination, the patient was a thin woman, with a protuberant abdomen and an increased respiratory rate. She was non-toxic in appearance. The vital signs were: temperature of 36.5°C (97.6°F); heart rate 105 beats per minute; blood pressure 123/85 mm Hg; respiratory rate 28 breaths per minute; and an oxygen saturation of 96% on room air. Pupils were equally reactive, and sclerae were icteric. The mucous membranes were moist and appeared jaundiced. Jugular venous distention was measured at 12 cm. The thyroid gland was not palpable. There were crackles halfway up the lung fields bilaterally, and the cardiac examination revealed a tachycardic rate, regular rhythm, and a three of six holosystolic murmur radiating to the axilla. The murmur was loudest at the apex and was noted to be intermittent. The abdomen was distended, soft, and nontender. There was a fluid wave, and the liver was percussed to 13 cm. The rectal examination revealed good tone, no tenderness, and the hemoccult test was negative. There was 2⫹ pitting edema to the knees, and good distal pulses. There were no rashes. The patient was alert and oriented on neurologic examination, with normal reflexes, gait, and cranial nerves.
Case Presentations of the Harvard Emergency Medicine Residency is coordinated by David F. M. Brown, Eric S. Nadel, MD, of the Harvard Medical School, Boston, Massachusetts
RECEIVED: 23 October 2000; ACCEPTED: 3 November 2000 153
MD,
and
154
A. M. Kumar et al.
Figure 1. EKG reveals sinus tachycardia with no evidence of ischemia.
Dr. Laura Bontempo: I agree that the weight loss is a concern for carcinoma. In light of the findings of painless jaundice, I am particularly concerned about a gastrointestinal malignancy blocking the biliary tree outlet. Had she noticed the jaundice or a change in urine color? Dr. David Brown: Congestive heart failure in a young patient with a new heart murmur has to raise the possibility of endocarditis. Had she had any recent dental work? Although the patient has no fever or peripheral signs of endocarditis, blood cultures should be drawn. Dr. Kumar: The patient noticed her eyes becoming yellow over the past 2 weeks. She had not noticed a change in urine color, although she did note a decreased urine output. She has not had any recent dental work. Dr. J. T. Nagurney: The presentation is most consistent with right and left-sided heart failure. Valvular heart disease, supported by the mitral regurgitation murmur, is most likely, but one also should consider dilated cardiomyopathy secondary to alcohol, cocaine, viral, hyperthyroid, or ischemic etiologies. Less likely causes of CHF include constrictive pericarditis and infiltrative disease such as amyloidosis and sarcoidosis. Chagas disease
might be considered if the patient had traveled to South America. Dr. Nadel: Are there any thoughts as to priorities in the management of this patient in the ED? Dr. Ron Walls: The most important thing is to establish that there is no immediately life-threatening process and, based on the presentation, this does not appear to be the case. In the ED, the patient needs a complete blood count (CBC) drawn to evaluate for anemia and eosinophilia, a metabolic panel looking for renal dysfunction or electrolyte abnormalities, an electrocardiogram (EKG), and a chest radiograph (CXR). I would draw three sets of blood cultures, separated in time and location, to evaluate for endocarditis. As far as treatment, I would administer furosemide intravenously. Dr. Bontempo: Given the painless jaundice, I would also consider an imaging test to look at the hepatobiliary system. You could proceed with an ultrasound or a computed tomography (CT) scan. The CT scan would give more information regarding other abdominal pathology, but the ultrasound would be better for assessing biliary tree dilatation. What did you do?
Congestive Heart Failure
155
Figure 3. Abdominal CT scan reveals ascites.
Figure 2. Chest X-ray reveals pulmonary edema and cardiomegaly.
Dr. Kumar: The patient was placed on a cardiac monitor, and the laboratory tests mentioned above were obtained, as well as tropinin I for the possibility of silent infarction, and a thryroid-stimulating hormone level was sent. The EKG (Figure 1) showed sinus tachycardia and right axis deviation. The CXR (Figure 2) revealed pulmonary edema and cardiomegaly. Laboratory values showed normal electrolytes and renal function and normal complete blood count with a hematocrit of 36.6% and white blood cell count of 6.0 K/mm3 with normal differential. The liver function panel revealed an alkaline phosphatase of 94 U/L, ALT 18 U/L, AST 34 U/L, and total bilirubin 3.6 mg/dL. The troponin-I was 0.05 ng/ mL. An abdominal/pelvic CT scan was performed (Figure 3), showing massive ascites, but no other intrabdominal pathology. Dr. Brown: Without evidence of pathology in the abdomen on CT scan, the ascites is likely secondary to hepatic congestion due to heart failure. Cardiac pathology, including endocarditis and an infiltrative process, must remain high on the differential diagnosis. As an inpatient, an echocardiogram should be obtained to search for valvular vegetations and to assess the ejection fraction. Rheumatic fever is a known risk factor for endocarditis, as is intravenous drug use. Is there any such history, and did you start antibiotics? Dr. Kumar: The patient denied any history of intravenous drug use. There was no known history of rheumatic fever. After blood cultures were drawn, vancomycin and gentamicin were given in the ED. The antibiotics
were targeted to cover Streptococcus viridans and Staphylococcus aureus. After the initiation of antibiotic therapy and diuretics, the patient was admitted to the medical service for further evaluation. A transthoracic echocardiogram revealed an ejection fraction of 57%, mild left ventricular enlargement, moderate aortic insufficiency, severe mitral regurgitation, and an echodensity on the posterior leaflet of the mitral valve. (Figure 4) The findings were suggestive of a chronic process, but endocarditis could not be ruled out. Antibiotics were continued until all six bottles of blood cultures remained negative for 48 h. Laboratory studies were directed at viral and rheumatologic causes of the valvular damage, and presumed chronic vegetation. Additional laboratory tests included an erythrocyte sedimentation rate (ESR), antistreptolysin-O (ASLO), and antinuclear anitibody (ANA). HACEK blood cultures were sent as well. These blood cultures test for Haemophilus species other than influezae as well as Actinobacillus, Cardiobacterium, Eikenella, and Kingella species. They account for up to 5% of patients with endocarditis and require special media in a carbon dioxide atmosphere for incubation. They are commonly referred to as the HACEK organisms (1). The ESR returned at 11, the ANA and ASLO were both negative, and the HACEK blood cultures were also negative. The patient continued to receive diuretics throughout her evaluation. The patient was brought to the operating room on hospital day 7 for a mitral valve replacement and aortic valve repair. Intra-operatively, the aortic valve was felt to have too much damage for successful repair, and both the mitral and aortic valves were replaced with prostheses. The removed valves were sent to the microbiology laboratory for cultures, which
156
A. M. Kumar et al.
Figure 4. Echocardiogram reveals an echodensity (mass) on the posterior leaflet of the mitral valve.
were negative. Pathology evaluation showed evidence of high-grade spindle cell sarcoma with muscle differentiation suggestive of a primary cardiac sarcoma. A Resident: Is jaundice common in patients with right heart failure? Dr. Brown: In moderate to severe heart failure, scleral icterus may be due to hepatic congestion. In this case, there also may be some contribution to the total bilirubin due to hemolysis on the abnormal heart valves. Dr. Nadel: This is obviously a rare diagnosis, not often seen in the ED. Please tell us more about it. Dr. Kumar: Cardiac tumors present with a wide array of symptoms mimicking the more common forms of heart disease, including chest pain, dysrhythmias, heart failure, syncope, pericarditis, or cardiac tamponade (2). Cardiac myxomas, although rare, are the most common type of cardiac tumor. They are histologically benign but still are life-threatening because of the possibility of obstruction, valvular damage, or embolus. Cardiac sarcomas are even rarer. They generally cause a rapidly progressive clinical deterioration over weeks to months with hemodynamic compromise, local invasion, or distant metastases (3). Diagnosis of cardiac tumors is best done by echocardiography, although differentiation of the type of tumor must be done by pathology. Not surprisingly, these tumors are often mistaken for endocarditis or collagen vascular disease. Cardiac magnetic resonance imaging can also better characterize the size, shape, and surface characteristics of the tumor (4). Ther-
apy is more successful for cardiac myxomas if they are detected early. Treatment involves surgical excision. Sarcomas generally have spread too far by the time of clinical presentation for surgical excision (5). There are a few case reports of palliation with the combination of surgery, radiotherapy, and chemotherapy, but results are generally poor. A few centers are experimenting with cardiac transplantation as a possibly curative measure for sarcomas (6). Dr. Bontempo: What was the patient’s post-operative course, and what is her long-term treatment plan? Dr. Kumar: The patient developed third-degree anterior-ventricular block on post-operative day 2 and had a pacemaker placed 2 days later by the electrophysiology service. A repeat echocardiogram showed an ejection fraction of 47%. The patient was evaluated by the oncology service and was offered palliative chemotherapy with adriamycin, and a referral to a cardiac transplant center for possible cardiac transplant. The rest of her hospitalization was uneventful, and the patient was discharged home on post-operative day 12.
REFERENCES 1. Meyer DJ, Gerding DN. Favorable prognosis of patients with prosthetic valve endocarditis caused by gram-negative bacilli of the HACEK group. Am J Med 1998;85:104. 2. Pucci A, Gagliardotto P, Zanini C, Pansini S, di Summa M, Mollo F. Histopathologic and clinical characterization of cardiac myx-
Congestive Heart Failure oma: review of 53 cases from a single institution. Am Heart J 2000;140:134 – 8. 3. Pins MR, Ferrell MA, Madsen JC, Piubello Q, Dickersin GR, Fletcher CD. Epithelioid and spindle-celled leiomyosarcoma of the heart. Report of 2 cases and review of the literature. Arch Pathol Laboratory Med 1999;123:782– 8. 4. Grebenc ML, Rosado de Christenson ML, Burke AP, Green CE,
157 Galvin JR. Primary cardiac and pericardial neoplasms: radiologicpathologic correlation. Radiographics 2000;20:1073–103; quiz 1110 –1, 1112. 5. Schaff HV, Mullany CJ. Surgery for cardiac myxomas. Seminars Thoracic Cardiovascular Surg 2000;12:77– 88. 6. Michler RE, Goldstein DJ. Treatment of cardiac tumors by orthotopic cardiac transplantation. Semin Oncol 1997;24:534 –9.